Considering the risk of sudden cardiac death (SCD) in patients with PRKAG2 mutations and the patient’s ventricular tachyarrhythmia, we recommended implantation of an implantable cardioverter-defibrillator (ICD) following detailed discussion with the patient and his relatives, and advised family screening for PRKAG2 mutations. Here, PRKAG2 is linked to Schnyder corneal dystrophy.